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All issues > Volume 50(7); 2007

Case Report
Korean J Pediatr. 2007;50(7):694-697. Published online July 15, 2007.
A familial case report of paroxysmal kinesigenic dyskinesia in three brothers
Oh Dae OD Kwon1, Sung Jin SJ Hwang2, Jun Hwa JH Lee2, Ji Eun JK Kim1, Kyung Jib KK Kim1, Eul Ju EJ Seo3
1Department of Neurology, School of Medicine, Catholic University of Daegu, Daegu, Korea
2Department of Pediatrics, College of Medicine, Sungkyunkwan University, Masan Samsung Hospital, Masan, Korea
3Department of Laboratory Medicine, University of Ulsan College of medicine and Asan Medical Center, Seoul, Korea
Correspondence Jun Hwa JH Lee ,Email: ljh3643@hanmail.net
Abstract
Paroxysmal kinesigenic dyskinesia (PKD), previously referred to as movement-provoked seizures, is a rare neurological condition that is characterized by short duration dystonic or choreoathetotic movements precipitated by sudden movement, a change in position or hyperventilation. It can be difficult to distinguish this syndrome from seizures. We reported on three brothers in one family all of whom developed abnormal involuntary dystonic or choreoathetotic movement with a tingling or stiffness sensory aura. Evaluations of the patients included general physical examinations, endoclinologic, metabolic studies, chromosomal analysis, video electroencephalograms and brain MRI imaging. All of these studies were normal except for an arachnoid cyst found in one patient. All symptoms showed excellent response to oxcarbamazepine (Trileptal ) or carbamazepine. Use of the video electroencephalogram can help differentiate familial PKD from seizures.

Keywords :Familial, Kinesigenic, Dyskinesia, Oxcarbamazepine (Trileptal )

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